Ganglioneuroma is an uncommon benign tumor and arise from neural crest cells or sympathetic and peripheral nerves. A 13-year-old boy was referred to our institute and presented with three-day history of right upper extremity weakness associated with neck pain for nearly one month. Magnetic resonance imaging showed right side dumbbell shaped extramedullary lesion at the C2-3 level extending outside through neural foramen. Paraspinal and posterior neck mass, cafe au lait spot was identified. We performed surgical decompression of the spinal cord by resection of intradural portion mass. Histopathological diagnosis was ganglioneuroma.